Every person has two adrenal glands that sit atop the kidney: one on the left and one on the right. Adrenal tumors can be broadly characterized by whether they are functioning or non-functioning, benign or malignant, or one-sided or both-sided.
From a functioning perspective, the adrenal glands make two general types of hormones. The outer part of the adrenal gland makes cortisol and cortisol-like hormones. The central portion of the gland makes adrenaline or adrenaline-like hormones. Functioning tumors over-produce these hormones, which can lead to problems such as uncontrolled blood pressure, electrolyte imbalances, and elevated glucose levels – this predisposes patients to significant risks such as cardiovascular disease and stroke. Examples of functioning tumors include:
• Cortisol-secreting tumor
• Sex-hormone secreting tumor
• Pheochromocytoma / Paraganglioma
From a cancer perspective, most adrenal tumors fortunately prove to be benign, and thus it follows that most functioning tumors and even some large tumors are benign. Notably though, many adrenal tumors are detected incidentally on imaging studies for other reasons and turn out to be non-functional. In these cases, surgery may be necessary if a mass appears suspicious for cancer; however, in less suspicious cases, active surveillance with follow-up imaging studies will be recommended to ensure early detection of cancer, should it develop. Examples of malignant adrenal tumors include:
• Adrenocortical carcinoma
• Adrenal metastases from another primary cancer (e.g. lung cancer)
• Malignant pheochromocytoma
It is important to understand that adrenal tumors can be either non-inherited (arise sporadically) or inherited (passed along the family tree). Some of the most common genetically inherited conditions involving adrenal tumors include:
• Multiple Endocrine Neoplasia Types IIA & IIB: these patients are risk for pheochromocytomas, and may have other tumors such as medullary thyroid cancer and hyperparathyroidism
• Von Hippel-Lindau (VHL): these patients are at risk for pheochromocytomas, and typically also develop tumors of the central nervous system, inner ear, kidney, neuroendocrine pancreas
• Li-Fraumeni Syndrome: these patients are at risk for adrenocortical carcinoma, as well as tumors of the breast, brain, and soft tissue (sarcomas)
• Hereditary paraganglioma-pheochromocytoma
At WCM, patients with inherited disorders, or suspicion for inherited disorders, are evaluated by our geneticists for thorough evaluation and counseling.
Comprehensive Diagnostic Evaluation
Here at the Weill Cornell Endocrine Oncology program, we will be providing you with cutting-edge diagnostic technology and innovative procedures to facilitate a rapid and definitive diagnosis. Imaging studies may include CT-scan, MRI, PET scan, MIBG, or Gallium-68 PET/CT DOTATATE scan, and blood tests will include screening for tumor markers that are found in your bloodstream. In some patients with functioning adrenal tumors, we offer additional testing to help determine which adrenal gland (right vs. left) is responsible for the hormone over-production. In this scenario, we collaborate with our colleagues in vascular surgery who perform the Adrenal Venous Sampling procedure. During this minimally invasive procedure, an expert vascular specialist inserts a catheter into a vein in your leg and is then able to obtain blood samples internally from the adrenal veins. The results of this procedure can help your endocrine surgeon determine which adrenal gland is diseased, and if surgery is right for you.
After we collect the pertinent information, you will be guided through the treatment process under the supervision of our world-renowned experts using a patient-centered multidisciplinary approach. At the Endocrine Oncology program, your doctor will help you choose the most appropriate treatment modality for you, which is centered on the most recent evidence-based medicine management protocols.
The majority of adrenal tumors are surgically removed, and here at Weill Cornell Medicine, we use minimally invasive techniques like laparoscopic surgery, robotically assisted surgery, or tumor-directed ablation that accelerates your recovery period and reduces length of stay in the hospital. Typically, patients who undergo minimally-invasive surgery are kept in the hospital only one night for observation and to ensure your remaining adrenal gland is functioning normally. Patients usually can resume most normal activities within a few days after minimally-invasive surgery – your surgeon will review any limitations with you before you go home.
Unique to adrenal surgery, patients with functioning tumors require close observation before, during, and after the operation. Pre-operatively, our surgeons carefully optimize your blood pressure, electrolyte levels, and glucose levels with medications to minimize the risk of complications during surgery. Intra-operatively, we work closely with a dedicated team of anesthesiologists who monitor you minute-by-minute during your operation. After surgery, as mentioned above, you will be kept in the hospital 1 to 2 days for monitoring. After being sent home, you will be enrolled in our post-operative surveillance program specific to your type of adrenal tumor.
Additional Treatments and Follow-up
After surgery, all patients with adrenal tumors are placed in a registry and followed long-term. This will entail blood work and imaging studies that our staff will monitor. Typically, patients with a history of adrenal tumors should have annual bloodwork to look for hormones and other biochemical markers that might suggest the return of the tumor or cancer; some patients will also require imaging studies as well.
In specific cases of malignant adrenal tumors, novel therapies and imaging technology provided by our physicians and scientists at our program will be used to optimize your outcome. Additional treatments for aggressive tumors may include radioisotope therapy, chemotherapy, or immunotherapy. Furthermore, several research protocols and molecular targeting techniques have been developed at our institution. Your doctor will discuss if you are a candidate for these protocols to more specifically target your adrenal tumor.
At the Weill Cornell Endocrine Oncology program, we are dedicated to a patient-centered multidisciplinary approach to provide you with the most up-to-date treatment options and access to clinical trials for adrenal tumors.